Natural history and control of epistaxis in a group of German patients with Rendu-Osler-Weber disease

Introduction: Epistaxis is the most common symptom of a complex, genetically determined vasculopathy, which is known under the notion hereditary hemorrhagic telangiectasia (HHT, Rendu-Osler-Weber-syndrome). This study was initiated to gain more knowledge about the natural history of epistaxis in a German HHT-population. Patients and methods: Data of 49 HHT patients were ascertained by interviewing these patients with a standardized disease specific questionnaire. Patientsfiles were retrospectively reviewed for data concerning age, gender, past medical history, laboratory parameters, num- ber of hospital admissions for epistaxis, conservative and operative types of therapy, treat- ment results and follow-up. Main result: Epistaxis was the first and most prominent symptom in 93% of the patients and could be triggered most frequently by stress. Half of the patients had experienced first episodes of epistaxis in childhood, but usually epistaxis did not become troublesome before the age of 35 years. The effects of hormonal changes or therapies with systemic hormones were inconclusive with regard to impact on epistaxis. Patients with septal perforations had to be admitted for inpatient epistaxis treatment more frequently than patients with an intact nasal septum. An overall reduction of frequency and intensity of epistaxis could be achieved in 89% of the patients through the daily use of nasal lubricants and a minimum of two treat- ment sessions with the Nd:YAG laser. However in none of the cases the treatment results were permanent. More than 50% of the patients, who had been screened for visceral arterio- venous malformations, were positive for pathologic vascular lesions. Principal conclusion: The natural history of epistaxis in German HHT patients is similar to previously described entities from other parts of the world. First clinical signs of HHT may be present at an earlier age than previously thought. SUMMARY